{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:05:28Z","timestamp":1790424328095,"version":"4.1.0"},"reference-count":44,"publisher":"American Society of Hematology","issue":"17","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,9,14]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Anti-A Disintegrin and Metalloproteinase with a ThromboSpondin type 1 motif, member 13 (ADAMTS13) autoantibodies cause a severe ADAMTS13 deficiency in immune-mediated thrombotic thrombocytopenic purpura (iTTP). ADAMTS13 consists of a metalloprotease (M), a disintegrin-like (D) domain, 8 thrombospondin type 1 repeats (T1-T8), a cysteine-rich (C), a spacer (S), and 2 CUB domains (CUB1-2). We recently developed a high-throughput epitope mapping assay based on small, nonoverlapping ADAMTS13 fragments (M, DT, CS, T2-T5, T6-T8, CUB1-2). With this assay, we performed a comprehensive epitope mapping using 131 acute-phase samples and for the first time a large group of remission samples (n = 50). Next, samples were stratified according to their immunoprofiles, a field that is largely unexplored in iTTP. Three dominant immunoprofiles were found in acute-phase samples: profile 1: only anti-CS autoantibodies (26.7%); profile 2: both anti-CS and anti-CUB1-2 autoantibodies (12.2%); and profile 3: anti-DT, anti-CS, anti-T2-T5, anti-T6-T8, and anti-CUB1-2 autoantibodies (8.4%). Interestingly, profile 1 was the only dominant immunoprofile in remission samples (52.0%). Clinical data were available for a relatively small number of patients with acute iTTP (&amp;gt;68), and no correlation was found between immunoprofiles and disease severity. Nevertheless, profile 1 was linked with younger and anti-T2-T5 autoantibodies with older age and the absence of anti-CUB1-2 autoantibodies with cerebral involvement. In conclusion, identifying acute phase and remission immunoprofiles in iTTP revealed that anti-CS autoantibodies seem to persist or reappear during remission providing further support for the clinical development of a targeted anti-CS autoantibody therapy. A large cohort study with acute iTTP samples will validate possible links between immunoprofiles or anti-domain autoantibodies and clinical data.<\/jats:p>","DOI":"10.1182\/bloodadvances.2020004172","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T14:59:17Z","timestamp":1631113157000},"page":"3427-3435","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":30,"title":["Anti-ADAMTS13 autoantibody profiling in patients with immune-mediated thrombotic thrombocytopenic purpura"],"prefix":"10.1182","volume":"5","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1808-6386","authenticated-orcid":false,"given":"Kadri","family":"Kangro","sequence":"first","affiliation":[{"name":"Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9078-9070","authenticated-orcid":false,"given":"Elien","family":"Roose","sequence":"additional","affiliation":[{"name":"Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B\u00e9rang\u00e8re S.","family":"Joly","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie biologique, H\u00f4pital Lariboisi\u00e8re, AP-HP Nord and;"},{"name":"EA3518, Institut de Recherche Saint-Louis, Universit\u00e9 de Paris, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5355-7318","authenticated-orcid":false,"given":"Gy\u00f6rgy","family":"Sinkovits","sequence":"additional","affiliation":[{"name":"Research Laboratory, Department of Internal Medicine and Hematology, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanja","family":"Falter","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine and;"},{"name":"Center for Thrombosis and Hemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Rheinland-Pfalz, Germany;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charis","family":"von Auer","sequence":"additional","affiliation":[{"name":"Center for Thrombosis and Hemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Rheinland-Pfalz, Germany;"},{"name":"Department of Hematology, Oncology and Pneumology, University Medical Center of the Johannes Gutenberg University, Mainz, Rheinland-Pfalz, Germany;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heidi","family":"Rossmann","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry and Laboratory Medicine and;"},{"name":"Center for Thrombosis and Hemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Rheinland-Pfalz, Germany;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marienn","family":"Reti","sequence":"additional","affiliation":[{"name":"Department of Haematology and Stem Cell Transplantation, Central Hospital of Southern Pest, National Institute of Haematology and Infectious Diseases, Budapest, Hungary;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Voorberg","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cellular Hemostasis, Sanquin-Academic Medical Center Landsteiner Laboratory, Amsterdam, The Netherlands;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zolt\u00e1n","family":"Proh\u00e1szka","sequence":"additional","affiliation":[{"name":"Research Laboratory, Department of Internal Medicine and Hematology, and MTA-SE Research Group of Immunology and Hematology, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4538-5154","authenticated-orcid":false,"given":"Bernhard","family":"L\u00e4mmle","sequence":"additional","affiliation":[{"name":"Center for Thrombosis and Hemostasis, University Medical Center, Mainz, Rheinland-Pfalz, Germany;"},{"name":"Department of Hematology & Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Coppo","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9s, Service d'H\u00e9matologie et Centre de R\u00e9f\u00e9rence des Microangiopathies Thrombotiques (CNR-MAT), H\u00f4pital Saint Antoine, Assistance Publique-H\u00f4pitaux de Paris, Paris, France; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agn\u00e8s","family":"Veyradier","sequence":"additional","affiliation":[{"name":"Service d\u2019H\u00e9matologie biologique, H\u00f4pital Lariboisi\u00e8re, AP-HP Nord and;"},{"name":"EA3518, Institut de Recherche Saint-Louis, Universit\u00e9 de Paris, Paris, France;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon F.","family":"De Meyer","sequence":"additional","affiliation":[{"name":"Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andres","family":"M\u00e4nnik","sequence":"additional","affiliation":[{"name":"Icosagen Cell Factory O\u00dc, Kambja vald, Tartumaa, 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